Microangiopathic Antiphospholipid Syndrome Research Articles

Микроангиопатический антифосфолипидный синдром в акушерской практике – риск генерализованной тромботической микроангиопатии

Микроангиопатический антифосфолипидный синдром в акушерской практике – риск генерализованной тромботической микроангиопатии

Microangiopathic antiphospholipid antibody syndrome due to anti‐phosphatidylserine/prothrombin complex IgM antibody

Herein we describe a case of microangiopathic antiphospholipid syndrome (MAPS) due to anti-phosphatidylserine/prothrombin complex (aPS/PT) IgM antibody successfully treated with rituximab. A significant correlation was observed between the clinical course and the aPS/PT IgM antibody titer, which can rise earlier before the appearance of clinical symptoms. Rituximab can be safely and effectively used for MAPS. Although detection of only aPS/PT IgM antibody is rare, aPS/PT IgM antibody might be associated with the pathogenesis of MAPS and might be a useful marker of disease activity.

Microangiopathic antiphospholipid antibody-associated syndrome in a pregnant lady

We describe a gravid 37-year-old Chinese lady with known triple positive primary antiphospholipid syndrome with previous recurrent deep vein thrombosis and early spontaneous miscarriages. She was managed with low-molecular weight heparin, aspirin, hydroxychloroquine, prednisolone and monthly intravenous immunoglobulin. She presented with recurrent per-vaginal bleeding at 22 weeks of gestation and was found to have abruptio placentae. Anti-coagulation was held off. She subsequently delivered a stillborn at 24 weeks and anti-coagulation was restarted. Day 5 post-delivery, she developed HELLP, with hemolytic anaemia (Hb 10.1 g/dL, haptoglobin <30 g/L, LDH 2206 U/L), elevated transaminases (AST 1196 U/L, ALT 1130 U/L) and thrombocytopenia (platelet 28 × 10^9/L). There were also episodes of acute severe headache and abdominal pain assessed to be secondary to microvascular ischemia as CT did not reveal any thrombosis. Her blood pressure hovered persistently above systolic 180 mmHg, and required at least three anti-hypertensives. These were coupled with a new onset proteinuria of 2 to 3 g/day. There was no evidence of disseminated intravascular coagulation. She was assessed to have microangiopathic antiphospholipid syndrome and was started on plasmapheresis. On Day 10 post-partum, the patient complained of foul-smelling vaginal discharge and was found to have retained products of conception, which was immediately evacuated. Her course was followed by poly-microbial sepsis secondary to Enterococcus fecalis, Klebsiella Pneumoniae and Escherichia coli. The patient was further treated with imipenem and she completed eight exchanges of plasmapheresis followed by five days of intravenous immunoglobulins with good clinical and biochemical improvement.

Microangiopathic antiphospholipid syndrome (MAPS) in the course of undifferentiated connective tissue disease

Introduction. The microangiopathic antiphospholipid syndrome (MAPS) is a subset of APS comprising those patients presenting with thrombotic microangiopathy and demonstrable antiphospholipid antibodies. Renal involvement occurs frequently in the course of MAPS with clinical symptoms of acute renal failure, hypertension, proteinuria and erythrocyturia. Case report. The report presents the patient with MAPS confirmed by kidney biopsy in the course of undifferentiated connective tissue disease.Conclusion. The authors emphasize the importance of effective anticoagulant treatment in order to inhibit thrombosis and renal damage in the course of MAPS.

Catastrophic antiphospholipid syndrome presenting with sudden renal failure: The lesson lies in vascular lesions.

Catastrophic antiphospholipid syndrome presenting with sudden renal failure: The lesson lies in vascular lesions.

Disseminated Intravascular Coagulation in the Presence of Antiphospholipid Antibodies: The Differential Diagnosis from Catastrophic or Microangiopathic Antiphospholipid Syndrome

Disseminated Intravascular Coagulation in the Presence of Antiphospholipid Antibodies: The Differential Diagnosis from Catastrophic or Microangiopathic Antiphospholipid Syndrome

Update on antiphospholipid antibodies: clinical significance

Antiphospholipid antibodies (aPL) are autoantibodies directed to phospholipid-binding proteins and classically related to thrombosis and pregnancy loss in the so-called antiphospholipid syndrome (APS). A wide spectrum of clinical manifestations have been observed in individuals with aPL. These may be asymptomatic, or they may exhibit clinical features suggesting APS, but not fulfilling the International Classification Criteria; typical APS manifestation in the absence of detectable aPL is defined as seronegative APS; microangiopathic APS may involve either one tissue only or may present with widespread thrombosis episodes accompanied by acute multi-organ failure in the catastrophic syndrome. The detection of different aPL profiles seems to increase the diagnostic specificity of the tests. The future research should focus on the development of assays that selectively identify aPL associated with increased risk of thrombosis or pregnancy complications, in order to lead to therapeutic strategies able to prev...

Microthrombotic/Microangiopathic Manifestations of the Antiphospholipid Syndrome

The paper presents an overview of clinical manifestations and histopathologic findings in different organs in microvascular thrombotic and microangiopathic antiphospholipid syndrome (MAPS). Subsets of antiphospholipid syndrome (APS) are presented and defined. Clinico-pathologic correlations seem insufficient so far, because of a lack of detailed systematic studies of the histopathology in different organs. Based on their own autopsy and biopsy studies, the authors propose a novel categorization of histopathologic lesions that occur in patients with classic and catastrophic APS. In addition to the already accepted category of a microvascular thrombotic type of lesions, microangiopathic lesions consistent with thrombotic microangiopathy are proposed to be included in new revised classification criteria for definite APS. Microvascular thrombotic and so far underestimated microangiopathic histopathologic lesions have been shown to appear in various combinations and of different ages in patients with both classic and catastrophic APS, which fits into the concept of MAPS. These preliminary findings of our studies are also in line with the most recent hypothesis of two main mechanisms in the pathogenesis of APS, emphasizing a key role of endothelial cell affection induced by aPL on the one hand and interference with coagulation cascade on the other side.

The catastrophic antiphospholipid syndrome and the microangiopathic antiphospholipid syndrome

The catastrophic antiphospholipid syndrome and the microangiopathic antiphospholipid syndrome